Fatal acute portal vein thrombosis associated with hepatic cysts in a patient with autosomal dominant polycystic kidney disease.

Autosomal dominant polycystic kidney disease (ADPKD) often involves polycystic liver disease (PLD). In severe cases, PLD can develop various complications. However, fatal acute portal vein thrombosis (APVT) associated with PLD has not been reported. A 64-year-old male reported mild consciousness disorder. He had been under maintenance hemodialysis for end-stage renal disease due to ADPKD with PLD. Because of recurring hepatic cyst infections, he had sustained high levels of C-reactive protein. Regarding the mild consciousness disorder, a diagnosis of hepatic encephalopathy was made based on an elevation of serum ammonia without any other abnormal liver function tests. Several days after his admission, hepatobiliary enzymes elevated, and acute liver failure progressed. Enhanced abdominal computed tomography suggested the possibility of complete occlusion of the portal vein by a thrombus. Based on an absence of obvious portosystemic collaterals, a diagnosis of APVT was made. The patient died 19 days after admission. Patients with PLD with repeated cystic infections have been seen to develop liver failure, and APVT formation may be one cause of the rapid progression of fatal liver failure. In conclusion, this is the first paper to report on the involvement of APVT in patients with PLD.

Neurological Presentations in Patients with COVID-19 in Cytokine Storm.

BACKGROUND: Coronavirus disease 2019 (COVID-19) infection causes a wide variety of neurological disorders by affecting both central and peripheral nervous systems. The cytokine storm (CS) has been blamed for the development of severe neurological disorders in COVID-19. However, the relationship between COVID-19 CS and neurological manifestations has not been adequately studied. Thus, we aimed to investigate the neurological presentations in patients with COVID-19 CS. METHODS: The study population consisted of hospitalized moderate-to-severe COVID-19 patients. It was divided into two groups CS (36 patients, 29.3%) and non-CS (87 patients, 70.7%) based on significant clinical symptoms, elevated inflammatory marker levels, radiological findings, and interleukin-6 levels (IL-6). RESULTS: The three most common neurological symptoms in the CS group were altered level of consciousness, headache, and unsteadiness. Altered level of consciousness was higher in the CS group (69.4%) than the non-CS group (25.3%) (p:0.001). The frequency of headache was comparable in both groups (p:0.186). The number of patients requiring intensive care unit and intubation was higher in the CS group (p:0.005 and p:0.001). The mortality rate in the CS group (38.9%) was higher than the non-CS group (8.0%) (p:0.001). IL-6, CRP, ferritin, neutrophil-lymphocyte ratio, procalcitonin, and D-dimer levels were higher in the CS group (for all p:0.001) while lymphocyte count was lower (p:0.003). CONCLUSION: The most common neurological presentation in patients with CS was altered level of consciousness. The presence of CS was an independent risk factor for high mortality.

IgG4-Related Disease Complicated by Brain Parenchymal Lesions Successfully Treated with Corticosteroid Therapy: A Case Report.

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is distinguished by the infiltration of IgG4-positive plasma cells in a variety of tissues and organs including the pancreas, salivary glands, retroperitoneal lesions, kidney, and lymph nodes with elevated serum IgG4 levels. Even so, central nervous system (CNS) lesions such as brain parenchymal lesions associated with IgG4-RD are scarce. So far, only six cases of IgG4-RD in relation with brain parenchymal lesions have been described, with its characteristics still being not clear. Here we have detailed a case of IgG4-RD with brain parenchymal lesions and reviewed previously-reported cases of IgG4-RD with brain parenchymal lesions. A 62-year-old Japanese male suffering from lung silicosis was admitted to our hospital for abdominal discomfort and altered consciousness. He has shown no major neurologic abnormalities except for drowsiness, urinary retention, and fecal incontinence. Brain magnetic resonance imaging has shown scattered hyperintense signals in the brain parenchyma. The serum IgG4 levels were elevated and systemic lymph nodes were enlarged. Biopsy from inguinal lymph nodes has shown massive infiltration of IgG4-positive plasma cells: the ratio of IgG4-positive/IgG-positive plasma cells was nearly 100%. Based on clinical courses, images, laboratory data, and pathological findings, a diagnosis of IgG4-RD that was complicated by brain parenchymal lesions and sacral nerve disturbance was confirmed. The patient was then given methylprednisolone pulse therapy (1g for 3 days) succeeding oral prednisolone (1 mg per body weight). The clinical and radiological improvements together with steroid therapy proposed IgG4-RD to be the cause of the lesions.

Assessing and treating pain in movement disorders, amyotrophic lateral sclerosis, severe acquired brain injury, disorders of consciousness, dementia, oncology and neuroinfectivology. Evidence and recommendations from the Italian Consensus Conference on Pain in Neurorehabilitation.

Pain is an important non-motor symptom in several neurological diseases, such as Parkinson's disease, cervical dystonia, amyotrophic lateral sclerosis, severe acquired brain injury, disorders of consciousness and dementia, as well as in oncology and neuroinfectivology. To overcome the lack of evidence-based data on pain management in these diseases, the Italian Consensus Conference on Pain in Neurorehabilitation (ICCPN) has defined criteria for good clinical practice among Italian neurorehabilitation professionals. Here a review of the literature (PubMed, EMBASE and gray literature) on pain characteristics, treatment and impact of pain in a neurorehabilitation setting is provided. Despite the heterogeneity of data, a consensus was reached on pain management for patients with these diseases: it is an approach originating from an analysis of the available data on pain characteristics in each disease, the evolution of pain in relation to the natural course of the disease and the impact of pain on the overall process of rehabilitation. There was unanimous consensus regarding the utility of a multidisciplinary approach to pain therapy, combining the benefits of pharmacological therapy with the techniques of physiotherapy and neurorehabilitation for all the conditions considered. While some treatments could be different depending on pathology, a progressive approach to the pharmacological treatment of pain is advisable, starting with non-opioid analgesics (paracetamol) and nonsteroidal anti-inflammatory drugs as a first-line treatment, and opioid analgesics as a second-line treatment. In cases of pain secondary to spasticity, botulinum neurotoxin, and, in some cases, intrathecal baclofen infusion should be considered. Randomized controlled trials and prospective multicenter studies aimed at documenting the efficacy of pain treatment and their risk-benefit profile are recommended for these conditions.

Muddy clinical waters: a missed pulmonary embolus.

The diagnosis of pulmonary embolism (PE) can be difficult to make. The consequences of missing a PE can be fatal and clinicians should always be vigilant. This case presents a patient admitted with fluctuations in consciousness and in a septic state. A preliminary diagnosis of aspiration pneumonia was made, and the patient went on to have an exploratory surgical procedure with no remarkable findings. Only after further investigation of the patient's fluctuations in consciousness was a retrospective diagnosis of a large PE made. The patient was anticoagulated and made a full recovery, with no outstanding pulmonary issues at follow-up 6 weeks after discharge. A prolonged immobile state, likely in part due to the patient's septic state and psychiatric history, along with recent surgery and hospitalisation were the most obvious risk factors.

[A case of possible neuro-Sweet disease with prolonged disturbance of consciousness and no dermal lesion during the course of dementia].

The patient was a 58-year-old man with 1-year history of cognitive decline, which was diagnosed as Alzheimer's disease in another hospital. He was admitted to our hospital for extreme fatigue, weight loss, and dysphagia, subsequent to the left peripheral facial paresis. Brain magnetic resonance (MR) imaging showed bilateral diffuse white matter lesions and hippocampal atrophy. After admission, he presented with sudden high fever, recurrent exacerbations of consciousness, and increased C-reactive protein level with marked neutrophilia, with the result that he underwent mechanical ventilation. Routine cerebrospinal fluid findings at the exacerbation were normal i.e. 4.7 cells/mm(3), 40 mg/dl of protein, but IL-6 concentration was mildly elevated to 22.2 pg/ml. After confirming the positivity of HLA (human leukocyte antigen) B54 and Cw1, we administered steroid to him and his physical activity and state of consciousness significantly improved. During the course of treatment, dermal lesion characterisitic of Sweet disease was absent. We diagnosed this case was possible neuroSweet disease proposed by Hisanaga in 2005.

High incidence of post-injury pneumonia in intensive care-treated trauma patients.

INTRODUCTION: Trauma patients are susceptible to post-injury infections. We investigated the incidence, as well as risk factors for development of pneumonia in intensive care unit (ICU)-treated trauma patients. In addition, we report pathogens identified in patients that developed pneumonia. METHODS: The study cohort consisted of 322 trauma patients admitted to the ICU at a level-one trauma centre following initial resuscitation. Patients 15 years or older with an ICU stay of more than 24 h were included. We investigated pre-hospital and hospital parameters during the first 24 h after admission and their possible association with pneumonia within 10 days of ICU admission. RESULTS: Majority of the patients were male (78%) and the median age was 41 years. The overall degree of injury was high with a median Injury Severity Score (ISS) of 24. Overall 30-day mortality was 9%. Eighty-five (26%) patients developed pneumonia during their first 10 days in the ICU. Univariate logistic regression revealed that intubation in the field, shock, Glasgow Coma Scale (GCS) 3-8, major surgery within 24 h after admission, massive transfusion and ISS > 24 were all risk factors for subsequent development of pneumonia. In the multivariable model, only GCS 3-8 was identified as an independent risk factor. In 42 out of the 85 cases of pneumonia, the diagnosis was defined by significant growth of at least one pathogen where Enterobacteriaceae and Staphylococcus aureus were the most common. CONCLUSIONS: Pneumonia is a common complication among ICU-treated trauma patients. Reduced consciousness is an independent risk factor for development of pneumonia after severe injury.

Minimum alveolar concentration: ongoing relevance and clinical utility.

Since its introduction in 1965, minimum alveolar concentration (MAC) has served as the standard measure of potency for volatile anaesthetic agents. It is defined as the minimum alveolar concentration of inhaled anaesthetic at which 50% of people do not move in response to a noxious stimulus. Within the last 20 years, it has been discovered that volatile anaesthetics inhibit mobility largely through action on the spinal cord, whereas the amnesic and hypnotic effects are mediated by the brain. Studies suggest that the concentration of volatile anaesthetic needed to prevent explicit memory from developing, and to produce unconsciousness, is usually substantially lower than the concentration required to prevent movement in response to surgery. This review highlights the contributions and limitations of MAC and its derivatives as metrics of anaesthetic potency with respect to particular behavioural outcomes. Recent evidence is presented suggesting that a protocol that alerts anaesthetists whenever MAC falls to < 0.5 or 0.7 has the potential to decrease intra-operative awareness with explicit recall, possibly to a similar extent as does a protocol based on processed electroencephalography-driven alerting.

Haematological, neurological and electrocardiographic findings in secondary hypothermia.

The association between secondary hypothermia and pancytopaenia is uncommon. A young woman presented with chronic hypothermia (28.8-34.6°C) secondary to surgical hypothalamic injury postcraniopharyingioma resection as a child. Associated findings included pancytopaenia (haemoglobin 8.1 g/dl, leucocytes 3500/mm(3), platelets 63,000/mm(3)), ataxia, upper motor neuron signs, decreased level of consciousness and new ECG changes. An extensive evaluation failed to reveal any cause of pancytopaenia other than chronic hypothermia. The haematological and neurological changes improved after active rewarming.

The reliability of nonreconstructed computerized tomographic scans of the abdomen and pelvis in detecting thoracolumbar spine injuries in blunt trauma patients with altered mental status.

BACKGROUND: Computerized tomography, traditionally utilized to evaluate and detect visceral abdominal and pelvic injuries in multiply injured patients with altered mental status, also has been useful for detecting thoracolumbar spine fractures and dislocations. The purpose of the present study was to test the reliability of nonreconstructed computerized tomography of the abdomen and pelvis as a screening tool for thoracolumbar spine injuries in blunt trauma patients with altered mental status. METHODS: The study consisted of fifty-nine consecutive patients with altered mental status who were admitted to a Level-II trauma center. Each patient had a nonreconstructed computerized tomographic scan of the abdomen and pelvis (5-mm slices), and of the chest when indicated, as well as anteroposterior and lateral radiographs of the thoracolumbar spine. Reconstructed computerized tomographic scans dedicated to the spine (< or =2-mm slices) were completed. With use of the reconstructions as the gold standard, sensitivity and specificity with 95% confidence intervals were calculated to assess the diagnostic accuracy of using the nonreconstructed computerized tomographic scans and the radiographs. RESULTS: Reconstructions of the spine detected seventy-two thoracolumbar spine fractures, whereas nonreconstructed computerized tomographic scans of the abdomen and pelvis detected fifty-eight and those of the chest detected sixteen. With use of the reconstructions as the standard, computerized tomography of the chest, abdomen, and pelvis had a sensitivity of 89% (95% confidence interval, 65% to 96%) and a specificity of 85% (95% confidence interval, 65% to 96%) for the detection of all fractures, compared with 37% and 76% for plain radiographs, respectively. Computerized tomography of the chest, abdomen, and pelvis was 100% sensitive and specific for the detection of whether a patient had any fracture at all, whereas radiographs were 54% sensitive and 86% specific. No fractures that were missed on nonreconstructed computerized tomography required surgery or other interventions. CONCLUSIONS: Nonreconstructed computerized tomography detected fractures of the thoracolumbar spine more accurately than plain radiographs did and is recommended for the diagnosis of thoracolumbar spine fractures in acute trauma patients with altered mental status. Reconstructions do not need to be ordered unless an abnormality that is found on the nonreconstructed computerized tomographic scan needs additional elucidation.

Experiences in the management of brainstem hematomas.

The purpose of this study was to present our experience in the management of spontaneous brainstem hematomas (BSH). Records of 58 consecutive patients were reviewed, including demographic data, symptoms, Glasgow Coma Scale, treatment, intraoperative findings (in surgical cases), and outcome according to the Glasgow Outcome Scale. Fifteen patients were comatose (GCS 4 or less): 11/15 patients were treated conservatively. Four patients with accompanying acute occlusive hydrocephalus were treated by placement of an external ventricular drainage. None survived. In nine patients (60%), arteriosclerosis and/or long-standing arterial hypertension were known and arteriopathic BSH was suspected. Forty-three patients were not comatose: 37 patients showed no impairment of consciousness (GCS 15), 6 patients presented with mild disturbance of conscious state (GCS 13), progressing to coma (GCS 8) in 1. In the majority (36/43) of the non-comatose patients (83.7%) cavernoma could be revealed and removed surgically. In six patients (14%), an atypically located arteriopathic BSH was assumed and treated medically. One patient had an underlying brainstem arteriovenous malformation and was treated radiosurgically. Many arteriopathic BSH cause immediate coma indicating direct and irreversible damage of midpontine structures. Thus, we suggest not to proceed to surgery, even if the bleeding is accompanied by acute hydrocephalus. The majority of BSH not resulting in immediate coma are caused by underlying cavernomas. In these cases surgery should be considered.

Image-guided resection of high-grade glioma: patient selection factors and outcome.

OBJECT: In patients with glioma, image-guided surgery helps to define the radiographic limits of the tumor to maximize safety and the extent of resection while minimizing damage to eloquent brain tissue. The authors hypothesize that image-guided resection (IGR) techniques are associated with improved outcomes in patients with malignant glioma. METHODS: Data recorded in 486 patients enrolled in the Glioma Outcomes Project were analyzed in this study. Demographic data and outcomes in patients who underwent IGR were compared with those in patients who underwent resection without IGR. Univariate analysis performed with chi-square testing was used to compare patient presentation, tumor characteristics, and death rates. Multivariate logistic regression was used to predict various outcome parameters. Patients who underwent IGR were younger and had smaller, lower-grade tumors than those in whom IGR was not performed. They were more likely to present with seizure and normal consciousness. Unexpectedly, gross-total resection was performed in significantly fewer patients with IGR than in individuals without IGR. Patients with IGR were more likely to be discharged home with the ability to live independently, and they had a shorter duration of hospital stay than patients without IGR. Survival was significantly longer in patients who underwent IGR, but multivariate analysis showed that glioblastoma multiforme (GBM) and age accounted for these observations. CONCLUSIONS: Selection bias occurs regarding patients who receive IGR; these biases include younger age, presentation with seizure and normal level of consciousness, tumor diameter less than 4 cm, and non-GBM on histopathological studies. Outcome appears to be improved in patients who undergo IGRs of high-grade gliomas. It is unclear if these improved outcomes are due to the selection of a more favorable patient population or to the IGR techniques themselves. It is likely that the full potential of image guidance in glioma surgery will not be realized until it is applied to a wider range of patients.

Failure of normal glycaemic regulation in a patient with severe hypothermia.

This report describes the case of an 88-year-old non-diabetic female who presented to the emergency department following a presumed hypoglycaemic collapse due to self-neglect. Subsequent rewarming and resuscitation demonstrated a number of the significant consequences of severe hypothermia, including apparent secondary impairment of glycaemic autoregulation. The phenomenon of reversible inhibition of insulin secretion due to severe hypothermia has been documented previously in the field of cardiac surgery. The hyperglycaemia was not treated with any antihyperglycaemic agent, and her recovery was uneventful. Subsequent blood sugar level monitoring was normal. If insulin is administered to the hypothermic patient, intensive monitoring of blood glucose is essential due to the increase in endogenous insulin secretion on rewarming.

Cough-induced hemiplegic migraine with impaired consciousness in cystic fibrosis.

The coughing paroxysms of patients with cystic fibrosis may occasion neurological symptoms. Although cough syncope is well-known, and is associated with headache and paralysis, a migrainous mechanism has not been reported. We reviewed the medical records, autonomic testing results, and responses to treatment in two cystic fibrosis patients with similar presentations of cough-induced impairment of consciousness followed by headache and paralysis. A 24-year-old woman and an unrelated 38-year-old man, both with cystic fibrosis, developed post-tussive neurologic deficits. Both patients reported infrequent dramatic spells, always preceded by major hemoptysis, and associated with left-sided paralysis, transient blindness, nausea, and severe pulsating headaches. Autonomic testing demonstrated only postural tachycardia and a near-vasodepressor episode in the woman, and mild, generalized sympathetic dysfunction in the man. Treatment for presumptive migraine with aura with verapamil nearly eradicated symptoms in both patients. Discontinuation of verapamil in the woman was associated with symptom recurrence and a stroke, with significant persistent residual left hemiparesis. In conclusion, cough-induced neurologic deficits were previously reported with cystic fibrosis, without clear understanding of the mechanism of impairment of consciousness. Based on the hemiparesis, nausea, and throbbing headache, which repeatedly followed the events in both patients, and based on the response to verapamil, we hypothesize a migrainous mechanism in both of our patients. The pathophysiology that links the hemoptysis to the spells deserves further investigation.

[Complications in home treatment of patients with severe disturbances of consciousness].

We have been providing home treatment for patients with severe disturbance of consciousness requiring various medical treatments for about 10 years. In the 65 cases we have encountered, we studied complications during the course of the home treatment. The proportions of infections, decubiti and convulsions were found to be very high. With infections in particular, conditions worsened rapidly in many cases, so we made it a principle to have the patients hospitalized early. We also investigated changes in serum albumin levels, peripheral blood lymphocyte count and prognostic nutritional index (PNI). Patients who develop infections repeatedly and succumb to early death often show low values for PNI and lymphocyte count. The low values are considered useful as one indicator of the general conditions of patients under home treatment. For the management of patients with severe disturbances of consciousness, it is important to keep a close liaison with other departments or hospitals and strengthen home nursing sections.